Chapter 9E. Severe myoclonic epilepsy in infancy (Dravet syndrome)

2004 ◽  
pp. 181-184
Keyword(s):  
Myoclonic Epilepsy ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy

Vagus Nerve Stimulation in Intractable Epilepsy Associated With SCN1A Gene Abnormalities

2017 ◽  
Vol 32 (5) ◽  
pp. 494-498 ◽  
Author(s):  
Stephen P. Fulton ◽  
Kate Van Poppel ◽  
Amy L. McGregor ◽  
Basanagoud Mudigoudar ◽  
James W. Wheless
Keyword(s):  
Vagus Nerve ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Seizure Control ◽  
Intractable Epilepsy ◽  
Myoclonic Epilepsy ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy ◽  
Scn1a Gene ◽  
Myoclonic Epilepsy Of Infancy

Mutations in the SCN1A gene cause a spectrum of epilepsy syndromes. There are 2 syndromes that are on the severe end of this spectrum. The classic severe form, Dravet syndrome, is an epileptic encephalopathy of childhood, causing cognitive decline as well as intractable seizures. Severe Myoclonic Epilepsy of Infancy–Borderline (SMEIB) is a term used to include cases with similar severities as those with Dravet syndrome, but lacking a single feature of classic severe myoclonic epilepsy of infancy. Vagus nerve stimulation is a nonpharmacologic treatment for intractable epilepsy. A retrospective review was conducted of patients with deleterious SCN1A mutations who had vagus nerve stimulation placement for treatment of their intractable epilepsy. These children had onset of their epilepsy between 3 and 29 months of age. Seizure control was assessed 6 months after implantation. Twenty patients are included in the study, with 12 implanted at our institution. Nine of the 12 patients implanted at our institution, who had confirmed pre- and post-implantation seizure assessments, showed improvement in seizure control, which was defined as >50% reduction in generalized tonic-clonic seizures, and 4 of those 12 reported improvement in cognitive or speech development. Seven of the 8 patients not implanted at our institution reported subjective benefit, with 4 relating “marked improvement” or seizure freedom. Vagus nerve stimulation appears to impart a benefit to children with deleterious SCN1A gene abnormalities associated with intractable epilepsy.

Dravet Syndrome (Severe Myoclonic Epilepsy in Infancy): A Retrospective Study of 16 Patients

2007 ◽  
Vol 22 (2) ◽  
pp. 185-194 ◽  
Author(s):  
Christian Korff ◽  
Linda Laux ◽  
Kent Kelley ◽  
Joshua Goldstein ◽  
Sookyong Koh ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Myoclonic Epilepsy ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy

Severe myoclonic epilepsy of infancy (Dravet syndrome): Recognition and diagnosis in adults

Neurology ◽  
2006 ◽  
Vol 67 (12) ◽  
pp. 2224-2226 ◽  
Author(s):  
F. E. Jansen ◽  
L. G. Sadleir ◽  
L. A. Harkin ◽  
L. Vadlamudi ◽  
J. M. McMahon ◽  
...  
Keyword(s):  
Myoclonic Epilepsy ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy ◽  
Myoclonic Epilepsy Of Infancy
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